Goodhill syndrome: a case report.

Conductive hearing loss with a normal tympanic membrane is a common reason for otolaryngology consultation, with otospongiosis being the most frequent cause and House syndrome being extremely rare, requiring systematic investigation. We report the case of a 31-year-old woman who presented with conductive hearing loss with a normal tympanic membrane. A temporal bone computed tomography (CT) scan confirmed a House-Goodhill syndrome due to fixation of the malleus head. Surgical intervention was considered to remove the attic bone synostosis with the malleus head, resulting in a significant clinical improvement. The Goodhill syndrome is a rare condition that causes hearing loss with a normal eardrum. The surgery can highly improve the hearing function.

Isolated Fracture of the Malleus: An Overlooked Cause of Conductive Hearing Loss?

OBJECTIVE: To report the largest case series of isolated malleus fractures with systematic review to characterize the disease's presentation and natural history, and provide suggestions for management. DATA SOURCES: PubMed, Embase, Cochrane Library. REVIEW METHODS: Retrospective cohort study was performed on 12 patients with isolated malleus fractures. History, physical exam, pre- and post-treatment audiograms, and imaging were obtained. Systematic review of the literature was performed. RESULTS: Including the cases herein, 58 isolated malleus fractures were identified, the majority of which were published in the 21st century. Mean time to presentation after injury was 34.4 months. Most common etiology was external auditory canal (EAC) manipulation. Physical exam and imaging did not identify any abnormality at presentation in 16% and 21% of cases, respectively. The majority of fractures involved the manubrium. Air-bone gap (ABG) at initial presentation ranged from 16 to 26 dB, and was greater at higher frequencies. Thirty-six cases underwent surgery. ABG improvement was greater at all frequencies for those who underwent surgery. Final ABG was significantly less than initial ABG at nearly every frequency for those who underwent surgery (p < 0.05), while not at any frequency for those who were observed. CONCLUSIONS: Isolated malleus fractures may occur more often than historical data suggests, and are perhaps underdiagnosed. Abrupt removal of a finger from the EAC with pain and hearing loss is nearly pathognomonic. Conductive hearing loss with ABG greater at higher frequencies is most often observed. Observation is unlikely to produce spontaneous improvements in hearing, while surgery demonstrates reliable decreases in ABG. Laryngoscope, 134:1032-1041, 2024.

Endoscopic ossiculoplasty in traumatic conductive hearing loss with intact tympanic membrane: a five-year experience.

BACKGROUND: Exploratory tympanotomy in cases of traumatic ossicular disruption with intact tympanic membrane is crucial for both diagnostic and therapeutic purposes. Performing this procedure using the endoscope is gaining popularity. Hence, this study aimed to demonstrate varieties of ossicular pathology and their management in our institution. METHODS: A retrospective evaluation was conducted of 136 ears in patients with traumatic ossicular disruption with an intact tympanic membrane, who underwent endoscopic exploratory tympanotomy. A proposed algorithm was followed, to incorporate different traumatic ossicular possibilities. Assessment of hearing outcomes and surgical complications was performed six months post-operatively. RESULTS: Incudostapedial dislocation was the most commonly encountered type of traumatic ossicular disruption (35.3 per cent). Air conduction threshold improved significantly following endoscopic ossiculoplasty, from 50.9 ± 6.35 dB pre-operatively to 22.35 ± 3.27 dB post-operatively, with successful air-bone gap closure. CONCLUSION: Endoscopic ear surgery is effective in the diagnosis and management of challenging cases of post-traumatic ossicular disruption with an intact tympanic membrane.

[Clinical application of retrosigmoid approach for BONEBRIDGE implantation after auricle reconstruction using expanded postauricular flap].

Objective: To explore the safety and reliability of retrosigmoid approach BONEBRIDGE implantation in patients with auricle reconstruction using skin expansion flap. Methods: A retrospective analysis was conducted on 43 congenital aural atresia cases (43 ears) who underwent BONEBRIDGE implantation from September 2019 to January 2023 in Beijing Tongren Hospital. 30 males and 13 females were included in this work. The implantation age was 9-36 years old (median age=10 y/o). All cases underwent auricle reconstruction surgery using the posterior ear flap expansion method, with 36 cases using the single expanded postauricular flap method and 7 cases using two-flap method. BONEBRIDGE implant surgery was performed during the third stage of auricle reconstruction or after all stages. The hearing improvements were evaluated by comparing the changes in pure tone hearing threshold and speech recognition rate of patients before and after BONEBRIDGE implantation. Routine follow-up was conducted to observe the hearing results and complications. SPSS 14.0 software was applied for data statistical analysis. Results: All 43 patients healed well and had no surgical complications when discharge. The average bone conduction hearing threshold after surgery was (8.2±6.6) dBHL, and there was no statistically significant difference compared to the preoperative [(8.1±5.7) dBHL] (P=0.95). After surgery, the threshold of hearing assistance with power on was significantly lower than that without hearing assistance [(32.8±4.6) dBHL vs (60.5±5.5) dBHL], and the difference was statistically significant (P<0.001). The speech recognition rate of monosyllable words, disyllabic words and short sentences in quiet environment increased to 72%, 84%, and 98% respectively. The differences were statistically significant (P<0.001). The speech recognition rate of monosyllabic words, disyllabic words, and short sentences in noise environment was significantly increased by 70%, 80%, and 92% respectively (P<0.001). After a follow-up of 4 to 47 months (median=24 months), the hearing results were stable and the aesthetic outcomes were satisfying. One patient had delayed hematoma around coil of the implant. After aspiration and compressed dressing for one week, hematoma was not recurrent. Conclusion: For patients after auricle reconstruction using expanded postauricular flap, the preference of retrosigmoid approach is a good choice in terms of safety and reliability of operation, as well as aesthetic appearance.

Acquired Stenosis of the External Ear Canal.

Acquired stenosis of the external ear canal (ASEEC) is a relatively uncommon condition. Stenosis or narrowing of the external ear canal (EEC) occurs lateral to the tympanic membrane resulting in a skin lined blind canal. Recurrent otorrhea, and conductive hearing loss are typical clinical features. Although ASEEC can be due to different etiologies, a common pathogenesis, namely an inflammatory cascade, has been implicated. Clinical evaluation, audiogram, and Computed tomography (CT scan) form the mainstay of diagnosis. Surgery is the primary modality for treatment. Restenosis is the most common postsurgical complication.

[Choristoma of the middle ear]. / Khoristoma srednego ukha i sostsevidnogo otrostka.

Choristoma is one of the varieties of congenital developmental anomalies, where one or another normal tissue of the body is located in an atypical place for itself. The short literary review of choristoma of middle ear is presented in article. A rare clinical cases of salivary gland choristoma of the middle ear (5-year-old girl with left-sided conductive hearing loss of III degree) and glial choristoma of the mastoid (19-year-old man with signs of chronic suppurative otitis media of the right ear) are described.

Audiological assessment with Matrix sentence test of percutaneous vs transcutaneous bone-anchored hearing aids: a pilot study.

PURPOSE: The study evaluated if there were differences between two types of bone-anchored hearing aids (BAHA), percutaneous vs transcutaneous implants in terms of audiological and psychosocial outcomes. METHODS: Eleven patients were enrolled. Inclusion criteria were: patients with conductive or mixed hearing loss in the implanted ear with a bone conduction pure-tone average (BC PTA) of the hearing threshold at 500, 1000, 2000, and 3000 Hz ≤ 55 dB HL, aged > 5 years. Patients were assigned to two groups: percutaneous implant (BAHA Connect) and transcutaneous implant (BAHA Attract). Pure-tone audiometry, speech audiometry, free-field pure-tone and speech audiometry with the hearing aid, and Matrix sentence test were performed. The Satisfaction with Amplification in Daily Life (SADL) questionnaire, the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire, and the Glasgow Benefit Inventory (GBI) were used to assess the psychosocial and audiological benefits provided by the implant, and the variation in the quality of life after the surgery. RESULTS: No differences were found comparing the data of Matrix SRT. APHAB and GBI questionnaires did not show a statistically significant difference comparing each subscale and the global score. The comparison of scores obtained from the SADL questionnaire demonstrated a difference in the "Personal Image" subscale with a better score for the transcutaneous implant. Furthermore, the Global Score of the SADL questionnaire was statistically different between groups. Other subscales did not show any significant difference. A Spearman's ρ correlation test was used to evaluate if the age could influence the SRT results; no correlation was found between age and SRT. Furthermore, the same test was used to confirm a negative correlation between SRT and the global benefit of the APHAB questionnaire. CONCLUSION: The current research confirms the absence of statistically significant differences comparing percutaneous and transcutaneous implants. The Matrix sentence test has shown the comparability of the two implants in the speech-in-noise intelligibility. Actually, the choice of the implant type can be done according to the patient's personal needs, the surgeon's experience, and the patient anatomy.

Effect of simulated acute bilateral severe conductive hearing loss on static balance function in healthy subjects: a prospective observational pilot study.

PURPOSE: Maintaining static balance is a process coordinated by central integration of visual, vestibular and somatosensory information. Whether or not hearing and spatial acoustic information contributes to the maintenance of static postural balance is unclear. METHODS: A prospective observational pilot study was performed. Twenty-five normal hearing adults (68% female; 19-31 years) underwent a computerized dynamic posturography test battery including the Sensory Organization Test (SOT), the Motor Control Test (MCT), and the Adaptation Test (ADT). The balance tests were performed two times, in a randomized sequence without or with acute hearing loss. Earplugs (sound insulation 37 dB) or headphones with white noise (sound volume 75 dB) induced the conductive hearing loss. Hence, all participants passed through four sequences of the balance test battery. A repeated-measures analysis of variance (ANOVA) was used to analyze the results. RESULTS: The ANOVA revealed no difference for any SOT and ADT subtest without hearing loss and simulated hearing loss (either earplugs or headphones; all p > 0.05). The ANOVA showed no longer latencies with simulated hearing loss compared to no hearing loss in both experiments with one exception: the reaction of the right foot during large forward translation was longer with hearing loss than without hearing loss in both experiments (p = 0.025). CONCLUSIONS: Overall, a simulated acute conductive bilateral moderate or severe hearing loss did not disturb the static balance function in normal hearing younger adults in this first small pilot study.

[Analysis of audiological features in infants with unilateral hearing loss].

Objective:To investigate the audiological characteristics and possible causes of unilateral hearing loss in infants and young children. Methods:105 infants from Beijing Maternal and Child Health Care Institution who failed the newborn hearing screening and were referred to the Children's Hearing Diagnosis Center of PLA General Hospital for hearing diagnosis. They were diagnosed with unilateral hearing loss and underwent clinical data collection. A full set of audiological examinations included ABR, 40 Hz auditory event related potential, ASSR, DPOAE, tympanometry. Results:①In initial diagnosis, 45 cases（42.86%） had mild hearing loss, 19 cases（18.10%） had moderate hearing loss, 14 cases（13.33%） had severe hearing loss, and 27 cases（25.71%） had severe hearing loss; Among them, 65 cases（61.90%） were conductive hearing loss or mixed hearing loss, and 40 cases（38.10%） were sensorineural hearing loss. ②83 of 105 cases had follow-up visits: 24 cases were normal, 15 cases with mild hearing loss, 4 cases with moderate hearing loss, 12 cases with severe hearing loss, and 26 cases with extremely severe hearing loss, 2 cases of hearing loss in both ears. ③From the initial diagnosis to the follow-up diagnosis, the change of mild hearing loss was the largest, followed by moderate hearing loss, severe and extremely severe hearing loss basically did not change; the number of mild and severe conductive hearing loss which recovered to normal hearing was most, the number of sensorineural hearing loss changed little. Conclusion:The infants who failed the newborn hearing screening and were diagnosed with unilateral hearing loss were mainly mild to moderate conductive hearing loss and severe to extremely severe sensorineural hearing loss. The hearing of children with hearing loss gradually improved, and severe and extremely severe sensorineural hearing loss remained unchanged.

[Etiology analysis and diagnosis of noninflammatory conductive hearing loss in children].

Objective:To investigate the etiology, diagnosis and treatment of noninflammatory conductive hearing loss in children. Methods:The clinical data of children patients admitted to the Eye & ENT Hospital of Fudan University from January 2019 to November 2022 were retrospectively analyzed. Results:A total of 179 cases（189 ears） were analyzed. The main symptoms from high to low were: ear tightness, hearing loss, earache, and facial paralysis. The degree of hearing loss was mild in 34 ears（19.5%）, moderate in 70 ears（40.2%）, moderate-severe in 52 ears（29.9%）, severe in 18 ears（10.3%）. The mean hearing threshold of otosclerosis was the highest（63.5±7.8） dB HL, and the mean air-bone gap of ossicular chain malformation was the largest（35.4±9.8） dB HL. The mean hearing threshold of the affected ear was（50.4±14.5） dB HL, and the mean air bone gap was（30.3±10.4） dB HL. After operation, the results were（36.1± 14.5） dB HL and（20.0±8.6） dB HL, respectively. Distribution of surgical methods for ossicular chain reconstruction: 88 ears（46.6%） of TORP, 49 ears（25.9%） of PORP, 8（4.2%） ears of Piston, 9 ears（4.8%） of autogenous ossicular reconstruction, and 35 ears（18.5%） of ossicular chain relaxation. CT diagnostic rate showed more sensitivity to malleus and incus abnormalities, the diagnosis rate of congenital middle ear cholesteatoma was the highest. The mean duration time of diagnosis was（2.2±2.9） years, while the diagnosis of ossicular chain malformation（[5.2±4.2]years）, otosclerosis（[4.4±4.1]years）, tympanosclerosis（[5.4±0.9]years） took longer. Conclusion:In the diagnosis and treatment of noninflammatory conductive hearing loss in children, the combination of detailed medical history, specialized examination and imaging examination can maximize the accuracy of diagnosis and achieve the purpose of personalized comprehensive treatment. Surgical intervention with appropriate timing is important to remove lesions and improve hearing.

[Evolution of auditory brainstem response in 0-6 years old normal hearing children and characteristics of children with abnormal acoustic conduction].

Objective:To explore the normal reference range of Click-ABR latency and interwave period in 0-6 years old children, and to analyze the clinical characteristics of Click-ABR in children with sound transmission function is abnormal. Methods:A total of 1791（3582 ears） normal hearing children aged 0-6 years and 176（258 ears） conductive hearing loss children were selected for Click-ABR. The differences of Click-ABR parameters in children of different months were analyzed, and the correlation between the degree of conductive hearing loss and Click-ABR parameters was explored. Results:The incubation period of wave Ⅰ was not correlated with the age of month, while the incubation period of wave Ⅲ, wave Ⅴ, waveⅠ-Ⅲ and wave Ⅰ-Ⅴ were highly correlated with the age of month. There was a positive correlation between the latency of wave Ⅰ and hearing threshold in the children with sound transmission function is abnormal under 80 dB nHL stimulation, and there was no difference between the standard values of wave Ⅰ-Ⅲ and Ⅰ-Ⅴ in the children with sound transmission function is abnormal and normal children. Conclusion:The latency of ABR wave Ⅲ and Ⅴ, and the interval between wave Ⅰ-Ⅲ and Ⅰ-Ⅴ shorten with the increase of age in children aged 0-6 years. The normal ABR values of children of different ages should be established in each hearing clinic for children as a reference. Combined with Click-ABR threshold and 80 dB nHL acoustic subwave Ⅰlatency, the abnormal conduction function can be preliminatively screened out, which should be further supplemented with other combinations of hearing diagnosis.

Diagnostic Value of the Air-Bone Threshold Gap in Stapes Fixation.

INTRODUCTION: The Carhart notch is a well-known sign of stapes fixation. However, previous studies have reported that the Carhart notch is not specific to stapes fixation and is also present in other middle ear diseases. Therefore, this study investigated the diagnostic value of threshold gap between air conduction and bone conduction (ABG) for stapes fixation, instead of the bone conduction dip representing the Carhart notch. METHODS: A total of 199 ears that underwent exploratory tympanotomy were enrolled in this retrospective study. They were categorized into three groups according to surgical findings: stapes fixation (SF), other ossicle fixation (OF), and chain disconnection (CD). Preoperative pure-tone audiograms and impedance audiograms were compared between the groups. RESULTS: The incidence of the Carhart notch did not differ between the groups. The ABG at 2,000 Hz showed a good diagnostic performance for distinguishing between the SF and CD groups (area under the curve, AUC = 0.816, p < 0.001), but poor performance for distinguishing between the SF and OF groups (AUC = 0.662, p = 0.003). Bone conduction at 2,000 Hz showed a moderate performance for distinguishing between the SF and CD groups (AUC = 0.707, p < 0.001) and did not show statistically significant results for distinguishing between the SF and OF groups (AUC = 0.594, p = 0.080). The tympanic membrane compliance was significantly higher in the CD group than in the SF group (p = 0.001). CONCLUSIONS: The Carhart notch was not a specific finding of SF. The sensitivity and specificity of ABG ≤15 dB at 2,000 Hz for distinguishing between SF and CD were 60.4% and 89.2%, respectively. To prepare for surgical interventions in patients with conductive hearing loss but a normal tympanic membrane, clinicians should comprehensively consider these results.

Middle ear effusion and newborn hearing screening.

PURPOSE: Middle ear effusion (MEE) is one of the reasons for screening failure and may require prolonged follow-up due to conductive hearing loss. We aimed to examine at 1-year follow-up, the fate of MEE. METHODS: From medical charts, computerized data were collected retrospectively of newborns born in the years 2012-2013 in Rambam Health Care Campus, Haifa city, Israel, who failed the Universal Newborn Hearing Screening (UNHS), and follow-up hearing evaluation data were extracted. RESULTS: Of 9527 newborns born in 2012-2013 in our institution, 144 [1.5%] failed the UNHS, and 46 were eventually diagnosed with conductive hearing loss caused by MEE. Spontaneous MEE clearance was recorded in 12 [26%], while 26 [57%] patients had persistent effusion that required further follow-up (10 [22%] required insertion of ventilation tubes and 16 [35%] were referred for further follow-up); 8 [17%] were lost to follow-up. CONCLUSION: Congenital MEE causing conductive hearing loss and UNHS failure is persistent and resolves at lower rates than non-congenital MEE.

[Role of computed tomography in the preoperative diagnosis of otosclerosis]. / Stellenwert der Computertomographie in der präoperativen Diagnostik der Otosklerose.

BACKGROUND: Otosclerosis is an osteodystrophy of the otic capsule and presents with progressive conductive hearing loss. Imaging studies, especially computed tomography (CT) and cone-beam CT, have gained increased relevance in the diagnosis of otosclerosis. OBJECTIVE: This study investigated whether there is a correlation between the extent of otosclerosis in high-resolution or cone-beam CT and hearing loss in pure-tone audiometry. MATERIALS AND METHODS: Based on an existing classification of otosclerotic foci, a classification was established. Preoperative CT scans of patients undergoing stapedotomy between 2015 and 2019 were evaluated and classified by two independent otorhinolaryngologists. The preoperative pure-tone audiograms were analysed and compared to the results of CT. RESULTS: A total of 168 CT studies (i.e., 168 ears) in 156 patients with intraoperatively confirmed otosclerosis were included in our study. A correlation between the extent of the otosclerotic focus or the calculated scores and hearing loss in pure-tone audiometry (air conduction, bone conduction and air-bone-gap) could not be proven. CONCLUSION: Preoperative CT is not obligatory. However, preoperative imaging using CT or cone-beam CT can be helpful to confirm the diagnosis and exclude other middle or inner ear pathologies as well as in planning of the surgical procedure in the overall context of otoscopy and audiometry. A correlation with the degree of hearing impairment could not be demonstrated and remains unclear.

Percutaneous Bone-Anchored Hearing Implant: Is It Clinically Useful in Korean?

BACKGROUND: The aim of this study is to investigate the clinical effectiveness of Ponto in Korea, a recently released percutaneous bone-anchored hearing implant. METHODS: 16 patients with single-sided deafness (SSD) and mixed or conductive hearing loss who underwent Ponto implantation from December 2018 to September 2020 were enrolled in the study. Puretone audiometry, the Korean version of the Hearing in Noise Test (K-HINT), sound localization test (SLT), and Pupillometry were performed pre- and three months post-operation. Standardized questionnaires, the Hearing Handicap Inventory for the Elderly (HHIE) and Speech, Spatial and Qualities of Hearing Scale (SSQ), were administered. RESULTS: The mean age of subjects was 55.5 (range, 48-67) years. Four males and 12 females participated in the study. The mean puretone average was 73.17 dB hearing level (HL) before surgery and significantly improved to 36.72 dB HL three months after surgery. The mean word recognition score improved from 26.0% to 90.75% after implantation. In the case of K-HINT, there was a significant difference in summation (Z = -2.250, P = 0.024) and head shadow effects (Z = -3.103, P = 0.002). There was no significant difference in root mean square error degree (RMSE) and hemifield identification scores for SLT testing. Pupillometry was performed to measure listening effort and the results revealed that the degree of pupillary dilatation decreased under the condition of quiet, 0 dB signal to noise ratio (SNR) and 3 dB SNR. The total score for HHIE decreased significantly (Z = -3.130, P = 0.002) while the SSQ score increased significantly (Z = -2.216, P = 0.027). CONCLUSIONS: The Ponto bone-anchored hearing system showed significant clinical benefit in Korean patients with conductive and mixed hearing loss and SSD.

A Deep Learning Approach to Predict Conductive Hearing Loss in Patients With Otitis Media With Effusion Using Otoscopic Images.

Importance: Otitis media with effusion (OME) is one of the most common causes of acquired conductive hearing loss (CHL). Persistent hearing loss is associated with poor childhood speech and language development and other adverse consequence. However, to obtain accurate and reliable hearing thresholds largely requires a high degree of cooperation from the patients. Objective: To predict CHL from otoscopic images using deep learning (DL) techniques and a logistic regression model based on tympanic membrane features. Design, Setting, and Participants: A retrospective diagnostic/prognostic study was conducted using 2790 otoscopic images obtained from multiple centers between January 2015 and November 2020. Participants were aged between 4 and 89 years. Of 1239 participants, there were 209 ears from children and adolescents (aged 4-18 years [16.87%]), 804 ears from adults (aged 18-60 years [64.89%]), and 226 ears from older people (aged >60 years, [18.24%]). Overall, 679 ears (54.8%) were from men. The 2790 otoscopic images were randomly assigned into a training set (2232 [80%]), and validation set (558 [20%]). The DL model was developed to predict an average air-bone gap greater than 10 dB. A logistic regression model was also developed based on otoscopic features. Main Outcomes and Measures: The performance of the DL model in predicting CHL was measured using the area under the receiver operating curve (AUC), accuracy, and F1 score (a measure of the quality of a classifier, which is the harmonic mean of precision and recall; a higher F1 score means better performance). In addition, these evaluation parameters were compared to results obtained from the logistic regression model and predictions made by three otologists. Results: The performance of the DL model in predicting CHL showed the AUC of 0.74, accuracy of 81%, and F1 score of 0.89. This was better than the results from the logistic regression model (ie, AUC of 0.60, accuracy of 76%, and F1 score of 0.82), and much improved on the performance of the 3 otologists; accuracy of 16%, 30%, 39%, and F1 scores of 0.09, 0.18, and 0.25, respectively. Furthermore, the DL model took 2.5 seconds to predict from 205 otoscopic images, whereas the 3 otologists spent 633 seconds, 645 seconds, and 692 seconds, respectively. Conclusions and Relevance: The model in this diagnostic/prognostic study provided greater accuracy in prediction of CHL in ears with OME than those obtained from the logistic regression model and otologists. This indicates great potential for the use of artificial intelligence tools to facilitate CHL evaluation when CHL is unable to be measured.

Congenital cholesteatoma: Clinical features and surgical outcomes.

OBJECTIVES: A typical presentation of congenital cholesteatoma (CC) is asymmetric conductive hearing loss (CHL). As CHL is usually associated with middle ear effusion, diagnosis of CC is frequently delayed. This study aimed to describe the clinical characteristics, treatment and outcomes of children with CC. METHODS: The medical files of children diagnosed with CC at a large tertiary pediatric medical center during 2000-2019 were reviewed. The primary outcome measures were: presenting symptoms, surgical findings, stage of disease, recurrence rate and hearing outcome. Imaging findings and the size of mastoid air cells were assessed in CT scans. RESULTS: Thirty-nine children were diagnosed with CC. The presenting symptom was unilateral CHL in 85%, with an average speech reception threshold of 41.5 ± 13.7 dB in the affected ear. The mean time from first symptoms to diagnosis was 1.3 years. The surgical approach was exploratory tympanotomy in 25% and canal wall up mastoidectomy in 69%. Seventy percent of the children presented with Potsic stage III-IV. The mean postoperative speech reception threshold was 26.4 ± 12.2 dB (P = 0.002). Recurrence of cholesteatoma occurred in 38% of the patients, mostly in stage III-IV. Mastoid air cell size was significantly smaller on the affected than the unaffected side. CONCLUSIONS: In children with persistent unilateral or asymmetric conductive hearing loss, CC should be suspected. Late diagnosis of CC is associated with a high recurrence rate. This highlights the need to promote awareness to the disease among primary physicians in the community health care system.

Automatic Prediction of Conductive Hearing Loss Using Video Pneumatic Otoscopy and Deep Learning Algorithm.

OBJECTIVES: Diseases of the middle ear can interfere with normal sound transmission, which results in conductive hearing loss. Since video pneumatic otoscopy (VPO) findings reveal not only the presence of middle ear effusions but also dynamic movements of the tympanic membrane and part of the ossicles, analyzing VPO images was expected to be useful in predicting the presence of middle ear transmission problems. Using a convolutional neural network (CNN), a deep neural network implementing computer vision, this preliminary study aimed to create a deep learning model that detects the presence of an air-bone gap, conductive component of hearing loss, by analyzing VPO findings. DESIGN: The medical records of adult patients who underwent VPO tests and pure-tone audiometry (PTA) on the same day were reviewed for enrollment. Conductive hearing loss was defined as an average air-bone gap of more than 10 dB at 0.5, 1, 2, and 4 kHz on PTA. Two significant images from the original VPO videos, at the most medial position on positive pressure and the most laterally displaced position on negative pressure, were used for the analysis. Applying multi-column CNN architectures with individual backbones of pretrained CNN versions, the performance of each model was evaluated and compared for Inception-v3, VGG-16 or ResNet-50. The diagnostic accuracy predicting the presence of conductive component of hearing loss of the selected deep learning algorithm used was compared with experienced otologists. RESULTS: The conductive hearing loss group consisted of 57 cases (mean air-bone gap = 25 ± 8 dB): 21 ears with effusion, 14 ears with malleus-incus fixation, 15 ears with stapes fixation including otosclerosis, one ear with a loose incus-stapes joint, 3 cases with adhesive otitis media, and 3 ears with middle ear masses including congenital cholesteatoma. The control group consisted of 76 cases with normal hearing thresholds without air-bone gaps. A total of 1130 original images including repeated measurements were obtained for the analysis. Of the various network architectures designed, the best was to feed each of the images into the individual backbones of Inception-v3 (three-column architecture) and concatenate the feature maps after the last convolutional layer from each column. In the selected model, the average performance of 10-fold cross-validation in predicting conductive hearing loss was 0.972 mean areas under the curve (mAUC), 91.6% sensitivity, 96.0% specificity, 94.4% positive predictive value, 93.9% negative predictive value, and 94.1% accuracy, which was superior to that of experienced otologists, whose performance had 0.773 mAUC and 79.0% accuracy on average. The algorithm detected over 85% of cases with stapes fixations or ossicular chain problems other than malleus-incus fixations. Visualization of the region of interest in the deep learning model revealed that the algorithm made decisions generally based on findings in the malleus and nearby tympanic membrane. CONCLUSIONS: In this preliminary study, the deep learning algorithm created to analyze VPO images successfully detected the presence of conductive hearing losses caused by middle ear effusion, ossicular fixation, otosclerosis, and adhesive otitis media. Interpretation of VPO using the deep learning algorithm showed promise as a diagnostic tool to differentiate conductive hearing loss from sensorineural hearing loss, which would be especially useful for patients with poor cooperation.

Tympanic membrane perforations: the importance of etiology, size and location.

PURPOSE: The ability to predict the degree of a conductive hearing loss caused by a tympanic membrane perforation is important for every otologist, as it may require additional diagnostic tests and prevent unexpected intraoperative findings. The aim of this study was to correlate the various characteristics of a perforation (etiology, size, location, involvement of the manubrium or umbo) with the degree and frequency predominance of the consequent hearing loss. METHODS: A transversal study in a tertiary hospital center was conducted between July 2019 and December 2020. Fifty-eight patients with 65 tympanic perforations underwent a comprehensive medical and audiological evaluation, which included an otoendoscopy. An image processing software (ImageJ®) was used to measure the perforated area. The qualitative variables were etiology, affected quadrants, presence of myringosclerosis and involvement of umbo or manubrium of the malleus. The air-bone gap was measured at 250, 500, 1000, 2000 and 4000 Hz. Primary outcomes (mean air-bone gap and pure-tone average) were evaluated to find clinical factors associated with worse hearing. RESULTS: Data collected from 50 ears was included. Perforation size showed a positive statistically significant correlation with the air-bone gap (r = .508; p < .001) and pure-tone average (r = .375; p < .001). Higher air-bone gaps were found in perforations involving the posterior quadrants and the manubrium (p < .001 and p = .031, respectively). Inflammatory causes showed higher bone and air conduction pure-tone averages (p = .031 and p = .084, respectively) compared to traumatic or iatrogenic. An "inverted V" pattern of the air-bone gap, with the 2 kHz frequency being the least affected, was a consistent finding. However, it was not due to the Carhart's notch in bone conduction. CONCLUSION: The conductive hearing loss resulting from a tympanic membrane perforation is etiology, size and location-dependent, with higher losses occurring for inflammatory backgrounds, large perforations and when the posterior quadrants or the manubrium are involved. If the "inverted V" is absent, additional middle ear pathology should be investigated.

Clinical and functional results after implantation of the bonebridge, a semi-implantable, active transcutaneous bone conduction device, in children and adults.

PURPOSE: Aim of the study was to evaluate the surgical, clinical and audiological outcome of 32 implantations of the Bonebridge, a semi-implantable transcutaneous active bone conduction implant. METHODS: In a retrospective cohort study, we analyzed data for 32 implantations in 31 patients (one bilateral case; seven age < 16 years) with conductive or mixed hearing loss, malformations, after multiple ear surgery, or with single-sided deafness as contralateral routing of signal (CROS). RESULTS: Four implantations were done as CROS. Five cases were simultaneously planned with ear prosthesis anchors, and 23 implantations (72%) were planned through three-dimensional (3D) "virtual surgery." In all 3D-planned cases, the implant could be placed as expected. For implant-related complications, rates were 12.5% for minor and 3.1% for major complications. Implantation significantly improved mean sound field thresholds from a preoperative 60 dB HL (SD 12) to 33 dB HL (SD 6) at 3 postoperative months and 34 dB HL (SD 6) at > 11 postoperative months (p < 0.0001). Word recognition score in quiet at 65 dB SPL improved from 11% (SD 20) preoperatively to 74% (SD 19) at 3 months and 83% (SD 15) at > 11 months (p < 0.0001). The speech reception threshold in noise improved from - 1.01 dB unaided to - 2.69 dB best-aided (p = 0.0018). CONCLUSION: We found a clinically relevant audiological benefit with Bonebridge. To overcome anatomical challenges, we recommend preoperative 3D planning in small and hypoplastic mastoids, children, ear malformation, and simultaneous implantation of ear prosthesis anchors and after multiple ear surgery.